Introduction

Budd–Chiari syndrome (BCS) is a rare condition affecting children. It is characterized by occlusion of venous outflow from liver at the level of hepatic veins (HV) or inferior vena cava (IVC). The management of BCS in children revolves around forming new collaterals for venous outflow or by elimination of blockage in the venous outflow tracts. These can be achieved by balloon venoplasty (BV), transjugular intrahepatic portosystemic shunting (TIPSS) or open shunt surgeries.

Aim

The aim of this study is to evaluate the management of BCS in children with balloon venoplasty as primary modality of treatment.

Materials and methods

This is a retrospective study which includes children diagnosed with BCS managed by balloon venoplasty by a single surgeon at a single institute. Once confirmed, the child was posted for balloon venoplasty and liver biopsy. When venoplasty was successful, child was subsequently heparinized and dose titrated. Routine follow up was mandated and dose adjustments were continued during follow up. In case of unsuccessful venoplasty, depending on the liver biopsy report, shunt procedure or liver transplantation is offered to patients. An algorithm was then designed for management of BCS in children.

Results

A total of 35 children who underwent evaluation of symptoms associated with Budd–Chiari syndrome were included in the study. Of all the children, 14 are alive and symptom free, 9 are deceased and 12 lost to follow up. Hepatic vein was the most common site of obstruction (85%), followed by both IVC and HV (15%). Overall, in 35 children, 26 had a successful balloon venoplasty, in 3 venoplasty was not done (2 spontaneous resolution and 1 died awaiting), in 6 it was unsuccessful (3 technical failures: 1 underwent TIPSS and 2 lost to follow up, 3 clinical failures: portocaval shunt for failed venoplasty).

Conclusion

Budd–Chiari syndrome is a rare condition affecting children. Balloon venoplasty as a primary modality of treatment for BCS is a promising option for management in children. Early and aggressive use of radiological intervention can help achieve recanalization in children.

Type of study

Clinical research paper.

Level of evidence

Level IV.

中文翻译：

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球囊静脉成形术是Budd-Chiari综合征儿童的主要治疗方式。

介绍

Budd–Chiari综合征（BCS）是一种影响儿童的罕见病。它的特征是在肝静脉（HV）或下腔静脉（IVC）的水平上阻塞了从肝脏流出的静脉。儿童中BCS的管理围绕形成新的静脉流出侧支或通过消除静脉流出道的阻塞来进行。这些可以通过球囊静脉成形术（BV），经颈静脉肝内门体分流术（TIPSS）或开放分流手术来实现。

目标

这项研究的目的是评估以球囊静脉成形术作为主要治疗方式的儿童的BCS管理。

材料和方法

这是一项回顾性研究，其中包括由单个机构的单个外科医师通过球囊静脉成形术诊断为患有BCS的儿童。一旦确认，该儿童将接受球囊静脉成形术和肝活检。静脉成形术成功后，随后对儿童进行肝素化并滴定剂量。要求进行常规随访，并在随访期间继续进行剂量调整。如果静脉成形术失败，则根据肝活检报告，为患者提供分流手术或肝移植。然后设计了一种用于儿童BCS管理的算法。

结果

该研究共包括35名接受与Budd-Chiari综合征相关的症状评估的儿童。在所有儿童中，有14名还活着并且没有症状，有9名死者，还有12名失踪。肝静脉是最常见的阻塞部位（85％），其次是IVC和HV（15％）。总体而言，在35例儿童中，有26例成功进行了球囊静脉成形术，在3例中未进行气囊成形术（2例自发解决，有1例等待死亡），在6例中未成功（3例技术失败：1例行TIPSS，2例失访，3例）临床失败：静脉腔成形术失败的门腔分流术。

结论

Budd–Chiari综合征是一种影响儿童的罕见病。球囊静脉成形术是治疗BCS的主要方式，是治疗儿童的有希望的选择。尽早而积极地使用放射干预可以帮助儿童实现再通。

学习类型

临床研究论文。

证据水平

第四级。